Persistent left superior vena cava is a rare congenital anomaly resulting from the failure of the left cardinal vein to degenerate in utero. This anomaly was discovered in 0.3% of large autopsy examinations. Its most relevant clinical implication is the association with cardiac impulse formation and conduction problems. In the case of DDD pacemaker implantation for this anomaly, skillful manual stylet shaping is required or an active fixation device, for example a screw device for stable positioning of pacemaker leads, is needed. We experienced a case of sick sinus syndrome in a patient with persistent left superior and absent right superior vena cava in 72-year-old man. We successfully implanted a DDD type pacemaker through the left superior vena cava via coronary sinus. This is the first Korean report of a successfully implanted permanent pacemaker in a patient with persistent left superior and absent right superior vena cava.